Orbital Sarcoidosis

Description of Ophthalmic-Orbital Sarcoidosis

Sarcoidosis is a disease of unknown aetiology characterized by chronic inflammation and granuloma formation across multiple systems of the body. Although it predominantly affects the lungs and eyes, it is seen to affect the skin, lymph nodes, liver, spleen, heart, central and peripheral nervous systems, musculoskeletal system, and salivary glands.

Pathophysiology of Ophthalmic-Orbital Sarcoidosis

Histologically widespread noncaseating epithelioid granulomata in the presence of classic clinical and radiolologic findings aid the diagnosis of sarcoidosis. It commonly presents with complications such as blindness, meningitis, arthritis, kidney disease, skin disease, other systemic involvement and even death. A pulmonologist’s consultation might be the only way to support a diagnosis of ophthalmic or neurosarcoid using a bronchoscopic guided biopsy of concomitant chest lesions.

Symptoms and Signs of Ophthalmic-Orbital Sarcoidosis

Sarcoidosis might present with isolated symptoms in specific systems, but more commonly it presents with a plethora of symptoms with varying severity and affecting multiple organ systems.

Ophthalmologic involvement might affect the optic nerve pathways or various structures within the orbit producing the following symptoms and signs:

Neuro-ophthalmic involvement

Diplopia: Due to cranial nerve palsies

Reduced vision with or without scotoma: Due to oedema or infiltration of the optic nerve

Cystoid macular edema (CME) is the collection of extracellular fluid in the outer plexiform layer of the retina. Intravenous fluorescein angiography (IVFA) might assist in diagnosis by demonstrating leakage of dye in and around the fovea.

Focal and discrete subretinal mottling on ophthalmoscopy

Fibrinous exudates in the pars plana

Orbital disease: 20% of patients with ophthalmic manifestations of sarcoidosis involve the orbit or the lacrimal gland presenting as a mass lesion with bulging of the eyes, drooping of eyelids, enlarged lacrimal gland or ophthalmoplegia.

Investigations form the basis of confirming a diagnosis of sarcoidosis due to the wide variety of eye disorders that mimic its manifestations. The various investigations that could lead to a diagnosis of sarcoidosis include:

Corticosteroids: They are the mainstay of treatment of ophthalmic and orbital sarcoidosis. Routes of administration include topical application, depot periocular injections, oral corticosteroids, and injectable corticosteroids. Immunosuppression and other adverse effects due to systemic corticosteroids should be discussed with the patient.

The treatment of choice for anterior uveitis is topical corticosteroids and might be used with cycloplegics.

Newer corticosteroids such as loteprednol etabonate are effective maintenance drugs having lesser incidence of steroid-induced glaucoma.

It is important to monitor improvement or progress of the disease with readjustments of steroid dosages to meet the requirements.

Retrobulbar Corticosteroid Injections: Depot or sub-Tenon corticosteroid injections subvert the inaccessibility to topical steroids in cases of intermediate and posterior uveitis. They are given weekly, bi-weekly, or monthly, up to 3 or 4 times.